Degradation of GABA

Stable Identifier
R-HSA-916853
Type
Pathway
Species
Homo sapiens
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GABA is metabolized in the mitochondrial matrix to succinate by the serial action of two enzymes, 4-aminobutyrate aminotransferase and suucinate semialdehyde dehydrogenase. Failure of the second reaction is associated with a rare human genetic disorder (Malaspina et al. 2016; Pearl et al. 2009).

Literature References
PubMed ID Title Journal Year
27311541 Succinic semialdehyde dehydrogenase deficiency (SSADHD): Pathophysiological complexity and multifactorial trait associations in a rare monogenic disorder of GABA metabolism

Malaspina, P, Roullet, JB, Pearl, P L, Ainslie, GR, Vogel, KR, Gibson, KM

Neurochem. Int. 2016
19172412 Succinic semialdehyde dehydrogenase deficiency: lessons from mice and men

Pearl, P L, Gibson, KM, Cortez, MA, Wu, Y, Carter Snead O, 3rd, Knerr, I, Forester, K, Pettiford, JM, Jakobs, C, Theodore, WH

J Inherit Metab Dis 2009
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